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Diabetic ketoacidosis (DKA) can quite frequently present in association with acute pancreatitis (AP) caused by transient severe hypertriglyceridemia (HTG). Here we report the case of a patient presenting with DKA, severe HTG and AP who received urgent plasma exchange for HTG control, and who reached adequate serum triglyceride levels only after appropriate DKA management. The treatment of patients presenting with DKA and coexistent AP associated with severe HTG should focus first on appropriate DKA management. Plasma exchange as a treatment for severe HTG in patients with DKA and AP should be evaluated carefully.